37 Pathogenesis of Tuberculosis of the Craniovertebral Junction: Its Implication in Surgical Management

10.1055/b-0034-81414

37 Pathogenesis of Tuberculosis of the Craniovertebral Junction: Its Implication in Surgical Management

Goel, Atul, Kataria, Naina Goel, Shah, Abhidha

Tuberculous involvement of the craniovertebral junction (CVJ) is still seen frequently in India and other developing countries. Tuberculosis (TB) of the CVJ usually occurs secondary to a primary focus elsewhere in the body. Osteoligamentous destruction and deformities leading to a range of clinical presenting symptoms have been recorded. An understanding of the site of involvement of TB in the CVJ, the pattern of its spread, and the nature of its pathogenetic effects on the osteoligamentous assembly is crucial for defining the management strategy. This chapter covers the staging of the disease, indications for surgery, and long-term outcome.

Natural Course of Disease

From our experience with the subject in general, and TB in particular, we identified the following stages of the disease, depending on the more commonly encountered pattern of bone and joint involvement. The cancellous part of the bone is most susceptible to TB. The cortical part is affected late, and the joints are secondarily involved.

**Fig. 37.1a–d A 25-year-old woman presented with complaints of severe neck pain for**∼**2 weeks. There was no neurological deficit. Investigations revealed that she had stage 1 tuberculosis (TB) of the craniovertebral junction (CVJ). She was treated with antituberculous drugs.** a Axial computed tomography (CT) scan shows destruction of the facet of the atlas. b Coronal view of CT scan showing destruction of the facet. The joints are normal, and there is no deformity. c Axial magnetic resonance imaging (MRI) showing the inflamma-tory reaction surrounding the facet. d Coronal MRI showing the affection of the facets and the adjoining region by inflammation.

Stage 1

In stage 1, there is unilateral involvement of the cancellous part of the facet of the atlas but no destructive deformation. Less frequently, there may be isolated and uni-lateral involvement of the cancellous part of the facets of the axis or of the odontoid process. Inflammatory granulomatous reaction is present, and caseous necrosis may be seen. Granulation tissue is usually located around the involved facet. The other parts of the atlas (or axis) bone and the contralateral facet are not involved ( Fig. 37.1 ).

In this stage, the patient experiences pain in the neck and restriction of neck movements. Systemic symptoms, such as loss of weight, loss of appetite, and fever, are usually present.

Stage 2

In stage 2, the disease progresses to involve the atlantoaxial joint by destructive necrosis and inflammation. The joint involvement is a result of extension of the inflammatory reaction. The destruction involves the atlantoaxial joint complex and extends to other parts of the atlas and/or axis bones. Tuberculous inflammation may extend widely. The contralateral joint is still unaffected in this stage. The incompetence of the joint and osseous destruction and the adjoining ligamentous disruption in such a situation have been known to result in atlantoaxial dislocation. The atlantoaxial dislocation is probably a result of the ineffectiveness of the alar and transverse ligaments, as their bone attachment site is destroyed. Because the contralateral atlantoaxial joint is normal, the atlantoaxial dislocation is of “fixed” and rotatory variety, and grossly mobile and reducible dislocation is seldom encountered. The facet of the atlas may be collapsed. Prevertebral or extradural spinal caseous necrosis or pus formation is usually encountered. On imaging, the joint space on the involved side is seen to be reduced or absent, whereas on the contralateral side, it is normal ( Figs. 37.2, 37.3, and 37.4 ).

**Fig. 37.2a, b An 18-year-old woman presented with complaints of severe torticollis and neck pain. She also had fever, generalized weakness, and malaise for**∼ **2 months. She responded well to antituberculous drugs. No surgery was done in this case.** a Axial MRI showing extensive caseous necrosis and destruction of the CVJ by TB. b Sagittal image of contrast-enhanced MRI showing the prevertebral extension of pus and involvement of the facets.

**Fig. 37.3a–c A 50-year-old woman presented with symptoms of torticollis and neck pain. There were no systemic symptoms. The patient was treated successfully with antituberculous drugs.** a Axial CT scan shows destruction of the facet of the atlas and the odontoid process. The contralateral facets are normal. b Axial CT scan with bone windows showing destruction. c Coronal CT scan showing destruction of the lateral mass of the atlas.

In this stage, the patient exhibits pain in the neck, neck muscle spasm, and severe restriction of neck movements. Torticollis is the characteristic and most prominent symptom. It appears to be a natural defense process, in which the neck turns to the contralateral side in an attempt to reduce all weight bearing by the affected lateral mass and the joint and protect the spinal cord from compression by infective granulation.

The patient may or may not have neurological symptoms or deficits.

Stage 3

In stage 3, the disease involves the contralateral atlanto-axial joint and other bones and joints in the region. Evidence of instability of the CVJ is usually seen ( Figs. 37.5 and 37.6 ).

In this stage, the patient usually has a neurologic deficit.

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